Origuchi N, Shigematsu H, Hatakeyama T, Nunokawa M, Yasuhara H, Muto T
First Department of Surgery, Faculty of Medicine, University of Tokyo, Japan.
Int Angiol. 1996 Mar;15(1):26-32.
The purpose of this report is to document what we have observed in patients with familial abdominal aortic aneurysms (FAAAs) between 1987 and 1993. Patients with FAAAs were reviewed and compared with those without familial clustering with regard to age, sex, past history, laboratory data, smoking habits, and type of implanted graft. We identified 7 families among which a total of 15 members had AAAs. The incidence of familial clustering reached 5.4%. The mean age of the FAAA group was significantly younger than that of the non-FAAA group (mean age: 65.8 +/- 10.3 versus 71.0 +/- 7.3 years). In the FAAA group, furthermore, patients in the second generation were significantly younger than those in the first generation (mean age: 55.3 +/- 10.5 versus 69.6 +/- 7.4 years). FAAA was significantly more often complicated by ischemic cardiac diseases. There were no significant differences in other risk factors. Interestingly, however, we observed a morphological similarity in the shape of the aneurysms within each family. Histological examinations showed moderate or severe lymphocytic infiltration into the aortic adventitia in 6 out of 9 cases. FAAA is clinically important, because it can represent a high-risk group that may benefit from a screening program for early detection and elective management of AAA. Studies of FAAAs will be useful for elucidating the pathogenesis of AAAs.
本报告的目的是记录我们在1987年至1993年间对家族性腹主动脉瘤（FAAA）患者的观察结果。对FAAA患者进行了回顾，并在年龄、性别、既往病史、实验室数据、吸烟习惯和植入移植物类型等方面与无家族聚集性的患者进行了比较。我们确定了7个家族，其中共有15名成员患有腹主动脉瘤。家族聚集发生率达到5.4%。FAAA组的平均年龄显著低于非FAAA组（平均年龄：65.8±10.3岁对71.0±7.3岁）。此外，在FAAA组中，第二代患者明显比第一代患者年轻（平均年龄：55.3±10.5岁对69.6±7.4岁）。FAAA更常并发缺血性心脏病。在其他风险因素方面没有显著差异。然而，有趣的是，我们观察到每个家族内动脉瘤形状存在形态学相似性。组织学检查显示，9例中有6例主动脉外膜有中度或重度淋巴细胞浸润。FAAA在临床上很重要，因为它可能代表一个高危群体，可能从腹主动脉瘤的早期检测和择期治疗筛查项目中受益。对FAAA的研究将有助于阐明腹主动脉瘤的发病机制。
